The effect of intravenous l-carnitine on propionic acid excretion in acute propionic acidaemia

1984 ◽  
Vol 143 (1) ◽  
pp. 61-63 ◽  
Author(s):  
H. B�hles ◽  
W. Lehnert
Keyword(s):  
Propionic Acid ◽  
Propionic Acidaemia ◽  
Acid Excretion

Inhibition of mitogen-activated proliferation of human peripheral lymphocytes in vitro by propionic acid

1999 ◽  
Vol 96 (1) ◽  
pp. 99-103 ◽  
Author(s):  
Moacir WAJNER ◽  
Kátia D. SANTOS ◽  
Jeanine L. SCHLOTTFELDT ◽  
Maureen P. ROCHA ◽  
Clóvis M. D. WANNMACHER
Keyword(s):  
Propionic Acid ◽  
Concanavalin A ◽  
Methylmalonic Acid ◽  
Cellular Growth ◽  
Propionic Acidaemia ◽  
Pokeweed Mitogen ◽  
Recurrent Infections ◽  
Peripheral Lymphocytes ◽  
Human Peripheral Lymphocytes

Recurrent infections are common features in patients affected by propionic acidaemia (McKusick 232000) and methylmalonic acidaemia (McKusick 251000). Since these disorders are biochemically characterized by tissue accumulation of propionic acid and methylmalonic acid respectively, it is possible that these compounds may act as immunosuppressants. We therefore investigated the effect of propionate and methylmalonate on cellular growth of human peripheral lymphocytes stimulated in vitro by phytohaemagglutinin, concanavalin A and pokeweed mitogen, a recognized test of cellular immunocompetence. Lymphocytes were cultured in flat-bottomed 96-well microplates at 37 ;°C for 96 ;h (phytohaemagglutinin and concanavalin A) or 144 ;h (pokeweed mitogen) in the presence of one mitogen at different concentrations and of one acid added at doses of 1.0, 2.5 or 5.0 ;mM. Cell blastogenesis was measured by the incorporation of tritiated thymidine into cellular DNA and compared with that of identical cultures with no acid added (controls). A consistent and progressive inhibitory effect of propionic acid with increasing concentrations in culture was identified with all mitogens and was more pronounced with pokeweed mitogen. Lymphocyte blastogenesis was not altered in the presence of methylmalonic acid. The effect of propionate was observed only when the drug was added at the beginning (phytohaemagglutinin-activated) or until 24 ;h (concanavalin A- and pokeweed mitogen-activated) of culture. The viability of lymphocytes after treatment with the drug, as assessed by the Trypan Blue exclusion test, revealed no change when compared with the same untreated lymphocytes, indicating no lymphocytotoxic activity. In conclusion, propionic acid, which accumulates in tissues of patients with propionic acidaemia, causes ‘in vitro’ immunosuppression, which may be related to the recurrent infections characteristic of these patients.

scholarly journals Applicability of an enzymatic quantitation of methylmalonic, propionic, and acetic acids in normal and megaloblastic states

Blood ◽  
1977 ◽  
Vol 49 (1) ◽  
pp. 125-137
Author(s):  
EP Frenkel ◽  
RL Kitchens
Keyword(s):  
Propionic Acid ◽  
Methylmalonic Acid ◽  
Vitamin B12 Deficiency ◽  
Normal Subjects ◽  
Methylmalonic Aciduria ◽  
Assay Method ◽  
Partial Purification ◽  
Etiologic Factor ◽  
Acid Excretion ◽  
B12 Deficiency

A rapid sensitive spectrophotometric assay for the measurement of methylmalonic and propionic acids in urine is described. The assay is based upon the quantitation of propionic acid using acetyl coenzyme A synthetase isolated from baker's yeast. This enzyme is highly specific for acetate and propionate, and acetate interference is eliminated by conversion to citrate. Methylmalonic acid was assayed by converting it to propionate by heat decarboxylation and then measuring the propionate increment over the endogenous amount in the noncarboxylated sample. Studies of urine obtained from normal subjects (by isolation, partial purification, and then assay by the isotope dilution technique) demonstrated urinary excretion of less than 1 mg of propionic acid and 1–5 mg of methylmalonic acid per day. In 22 consecutive patients with documented vitamin B12 deficiency, methylmalonic acid excretion in excess of 30 mg/24 hr was found. In four other patients, with only neurologic involvement methylmalonic aciduria aided in identifying B12 deficiency as an etiologic factor. Methylmalonic acid excretion was measured by direct assay of an aliquot of urine, requiring neither a valine load nor special extraction procedures. Propionic aciduria was variably increased in B12 deficiency and did not correlate either with the severity of the deficit or degree of methylmalonic aciduria. The assay was performed on urine, but it is potentially applicable to tissue extracts. In addition, this assay method can be utilized for the quantification of urine acetate levels as well.

scholarly journals ODD-NUMBERED LONG-CHAIN FATTY ACIDS IN ERYTHROCYTE MEMBRANE LIPIDS AND PROPIONIC ACID IN PLASMA FOR METABOLIC CONTROL OF PATIENTS WITH PROPIONIC ACIDAEMIA

1992 ◽  
Vol 32 (5) ◽  
pp. 628-628
Author(s):  
Wolfgang Sperl ◽  
Christian Murr ◽  
Ludwig Doczy ◽  
Andrea Mayr ◽  
Udo Wendel
Keyword(s):  
Fatty Acids ◽  
Metabolic Control ◽  
Propionic Acid ◽  
Erythrocyte Membrane ◽  
Membrane Lipids ◽  
Propionic Acidaemia ◽  
Long Chain Fatty Acids ◽  
Long Chain ◽  
Chain Fatty Acids

scholarly journals Metabolic responses to low doses of cyanocobalamin in patients with megaloblastic anaemia

1968 ◽  
Vol 22 (4) ◽  
pp. 575-582 ◽  
Author(s):  
J. F. Adams ◽  
R. Hume ◽  
Elizabeth H. Kennedy ◽  
T. G. Pirrie ◽  
J. W. Whitelaw ◽  
...  
Keyword(s):  
Propionic Acid ◽  
Serum Iron ◽  
Serum Vitamin ◽  
Megaloblastic Anaemia ◽  
Cobalamin Deficiency ◽  
Metabolic Responses ◽  
Vitamin B ◽  
Acid Excretion ◽  
Transient Rise ◽  
Abrupt Rise

1. The metabolic responses to daily treatment with 1 μg cyanocobalamin were studied in sixteen patients with megaloblastic anaemia due to cobalamin deficiency.2. The fall in serum iron was sluggish in most patients. The pattern of reticulocyte responses varied widely, the most common being a plateau with suboptimal maximal value.3. Urinary propionic acid excretion before treatment was normal in two out of six patients. A transient rise during treatment was observed in two patients, possibly owing to demands on coenzyme stores. The fall in urinary propionic acid excretion during treatment was slow.4. Serum vitamin B12levels rose during treatment. In two patients an abrupt rise was found, possibly owing to release of stored cobalamin into the circulation.

scholarly journals Applicability of an enzymatic quantitation of methylmalonic, propionic, and acetic acids in normal and megaloblastic states

Blood ◽  
1977 ◽  
Vol 49 (1) ◽  
pp. 125-137 ◽  
Author(s):  
EP Frenkel ◽  
RL Kitchens
Keyword(s):  
Propionic Acid ◽  
Methylmalonic Acid ◽  
Vitamin B12 Deficiency ◽  
Normal Subjects ◽  
Methylmalonic Aciduria ◽  
Assay Method ◽  
Partial Purification ◽  
Etiologic Factor ◽  
Acid Excretion ◽  
B12 Deficiency

Abstract A rapid sensitive spectrophotometric assay for the measurement of methylmalonic and propionic acids in urine is described. The assay is based upon the quantitation of propionic acid using acetyl coenzyme A synthetase isolated from baker's yeast. This enzyme is highly specific for acetate and propionate, and acetate interference is eliminated by conversion to citrate. Methylmalonic acid was assayed by converting it to propionate by heat decarboxylation and then measuring the propionate increment over the endogenous amount in the noncarboxylated sample. Studies of urine obtained from normal subjects (by isolation, partial purification, and then assay by the isotope dilution technique) demonstrated urinary excretion of less than 1 mg of propionic acid and 1–5 mg of methylmalonic acid per day. In 22 consecutive patients with documented vitamin B12 deficiency, methylmalonic acid excretion in excess of 30 mg/24 hr was found. In four other patients, with only neurologic involvement methylmalonic aciduria aided in identifying B12 deficiency as an etiologic factor. Methylmalonic acid excretion was measured by direct assay of an aliquot of urine, requiring neither a valine load nor special extraction procedures. Propionic aciduria was variably increased in B12 deficiency and did not correlate either with the severity of the deficit or degree of methylmalonic aciduria. The assay was performed on urine, but it is potentially applicable to tissue extracts. In addition, this assay method can be utilized for the quantification of urine acetate levels as well.

Role of Dietary Propionic Acid and Bile Acid Excretion in the Hypocholesterolemic Effects of Oligosaccharides in Rats

1994 ◽  
Vol 124 (4) ◽  
pp. 531-538 ◽  
Author(s):  
Marie-Anne Levrat ◽  
Marie-Laure Favier ◽  
Corinne Moundras ◽  
Christian Rémésy ◽  
Christian Demigné ◽  
...  
Keyword(s):  
Bile Acid ◽  
Propionic Acid ◽  
Acid Excretion ◽  
Bile Acid Excretion

Amino Acid Excretion in Patients With Gastro Intestinal Disease During Ingestion of Various Protein Supplements

1950 ◽  
Vol 16 (4) ◽  
pp. 757-763 ◽  
Author(s):  
A. Leonard Sheffner ◽  
Joseph B. Kirsner ◽  
Walter L. Palmer
Keyword(s):  
Amino Acid ◽  
Intestinal Disease ◽  
Acid Excretion ◽  
Protein Supplements

Propionic acid as a precursor in the biosynthesis of animal fatty acids

1961 ◽  
Author(s):  
E.J. Masoro ◽  
Edith Porter
Keyword(s):  
Fatty Acids ◽  
Propionic Acid
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